PMID: 8464784Mar 1, 1993Paper

Renal tubular dysgenesis: a description of early renal maldevelopment in siblings

Pediatric Pathology
R A MetzmanE A Dellers

Abstract

A family is described in which three siblings, born after pregnancies complicated by oligohydramnios, developed renal abnormalities. In the first infant, of 38 weeks gestation, histologic changes were nearly identical to those found in renal tubular dysgenesis (RTD), a recently identified disorder characterized by the absence of recognizable renal proximal tubules. Additional findings include bilateral renal vein thrombosis and marked calvarial bone hypoplasia. The other two gestations were 20 and 22 weeks long. Renal histology in these cases showed nonspecific abnormalities with focal tubular dilatation, decreased tubule formation, and increased interstitial connective tissue. Clearly recognizable proximal tubules were present, though decreased in number. The latter two gestations described herein are the earliest examined in a family with RTD and the renal abnormalities may represent early changes seen in this disorder.

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Citations

Nov 14, 1997·Journal of Medical Genetics·D E McFaddenS Langlois
Nov 1, 1995·Pediatric Pathology & Laboratory Medicine : Journal of the Society for Pediatric Pathology, Affiliated with the International Paediatric Pathology Association·I ArielE Rosenmann
Jun 23, 2006·Journal of the American Society of Nephrology : JASN·Mireille LacosteMarie Claire Gubler
Mar 17, 2004·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Vibha Jain, Debra Beneck
Dec 16, 1998·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·J S JohalC E Oyer
Dec 16, 1998·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·K C ObergE P Hawkins
Apr 11, 2003·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Monique E De PaepeFrancois I Luks
Nov 19, 2004·Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society·Jessica D de RooijRonald R de Krijger

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